Childhood disintegrative disorder (CDD), also known as Heller’s syndrome and disintegrative psychosis, is a rare condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills.
It can be frightening for parents when a child loses previously gained developmental skills. One cause of this skill loss is childhood disintegrative disorder (CDD). The condition is a rare childhood developmental disorder that is often mistaken for late-onset autism. While many symptoms are similar, there are important differences between this condition and other autism spectrum disorders. Early diagnosis is important to help children with CDD get the appropriate treatment and the best prognosis for a healthy life.
About Childhood Disintegrative Disorder
CDD History
Austrian special educator Theodore Heller discovered childhood disintegrative disorder, also known as Heller’s syndrome and disintegrative psychosis, in 1908. Heller introduced the condition as dementia infantilis to describe the sudden loss of language and social skills by young children. The medical community did not recognize the CDD diagnosis for years because many experts believed the symptoms were linked to other medical problems. It was not until after autism was discovered that the developmental disorder gradually emerged as a recognized neurological condition.
A Pervasive Developmental Disorder
CDD is a disorder that manifests in children ages 2 through 4, who begin to demonstrate severe loss of previously gained communications, language and social skills after years of normal development, says the Childhood Study Center at Yale University. According to the National Institute of Mental Health, CDD is rare, and affects less than two in 100,000 children, many of whom were previously diagnosed with autism.
The American Psychiatric Association’s Diagnostic Statistical Manual, Fourth Edition (DSM-IV), considers CDD a pervasive developmental disorder (PDD), along with the following conditions:
- Autism
- Asperger’s syndrome
- Rett’s syndrome
- Pervasive developmental disorder-not otherwise specified
The DSM-IV categorizes these conditions together because they are neurological disorders that affect a child’s development of normal language, communication and social skills. The DSM-V may merge CDD with autism, along with Asperger’s and Rett’s syndrome, in 2012.
Causes of CDD
At this time, the cause of CDD is unknown. Ongoing research point to a number of possible cause theories including possible genetic causes, environmental triggers, a malfunctioning autoimmune response, or brain abnormalities.
There are certain medical conditions associated with CDD, such as epilepsy, tuberous sclerosis, lipid storage diseases or subacute sclerosing panencephalitis. In fact, CDD may increase an affected person’s chance of developing epilepsy. However, the Mayo Clinic points out that there is not currently sufficient evidence that any of these conditions cause CDD.
The Difference Between CDD and Autism
Since CDD is so rare and resembles autism, correct diagnosis is sometimes difficult. Some children later diagnosed with CDD were first misdiagnosed with autism. Both CDD and autism share a similar set of language, communication, social and behavioral impairments. According to the National Institute of Mental Health, there have been studies suggesting that CDD may be a rare form of autism, which further complicated diagnosis. However, CDD has the following unique symptoms not shared by autism and other PDDs:
- Normal development for two or more years
- Sudden loss of previously acquired language, communications and social skills
- Previous potty training lost and child is no longer able to control his bladder and bowel
- Child begins to suffer seizures periodically
Symptoms
The onset of CDD is sudden and severe. Sometimes, the child may even comment on symptoms. The process of losing developmental skills can happen abruptly within a week or gradually increase in severity over several months. The symptoms of CDD include:
- Loss of previously learned communication skills, including vocabulary and language development
- Has limited or no language skills and cannot participate in a conversation
- May stop talking
- Unable to understand verbal and nonverbal communication
- No longer able to control the bladder and bowel
- Shows no interest in social interaction
- Cannot be comforted by parents
- Does not want to develop relationships with others
- Cannot understand the emotions of others
- No interest in pretend play
- Appearance of repetitive and obsessive behaviors
- Repeats irrational behavior in cycles, such as rocking, spinning and hand flapping
- Repeats words or phrases out of context
- Prefers strict routine
- Gets irrationally upset and may throw tantrums if the routine is interrupted
- Seizures develop
Treatment Options
While there is currently no known cure for CDD, there are effective interventions to improve quality of life. Treatment options include many of the therapies and medications that treat autism to encourage the development of language, communication and social skills. Helpful treatment options include the following:
- Applied Behavioral Analysis
- Speech and language therapy
- Occupational therapy
- Sensory integration therapy
- Facilitated communication
- Picture Exchange Communication System
Since there is not a particular CDD medication, doctors sometimes prescribe medication to treat specific symptoms. For example, doctors often prescribe anticonvulsant drugs to patients who suffer from epileptic seizures. People with CDD who experience aggressive or obsessive behaviors may be prescribed antipsychotic drugs or medication to treat anxiety and depression.
Conditions with Similar Symptoms
CDD has similar symptoms to other neurological problems. Conditions with similar symptoms include:
- Head trauma
- Childhood dementia
- Childhood schizophrenia
- Expressive language disorder
- Selective mutism
- Mental retardation
- Other autism spectrum disorders
A pediatrician will perform a comprehensive medical and mental evaluation to make an accurate CDD diagnosis and rule out other conditions.
How Families Can Get Help
Early intervention is essential to the best prognosis for healthy life with dignity. If CDD or any other medical condition is suspected, parents should contact a doctor immediately to seek a diagnosis.
Even though children with CDD often require lifelong care, current treatment options can help improve symptoms and overall life quality. Each case of CDD is individual and despite the dire outlook of the condition, there is no limit to how much any one child can improve with the right treatment plan.
To help the affected child, parents should thoroughly research the condition and available treatment options in order to find the right doctors, teachers and treatments for their children.
Support groups can help families cope and provide helpful local resources for CDD treatment. Support group information and additional resources are available at the Autism Society, National Autism Association and the Autism Help site.
